title: Complement therapy in atypical haemolytic uraemic syndrome (aHUS).
abstract: Central to the pathogenesis of atypical haemolytic uraemic syndrome (aHUS) is over-activation of the alternative pathway of complement. Inherited defects in complement genes and autoantibodies against complement regulatory proteins have been described. The use of plasma exchange to replace non-functioning complement regulators and hyper-functional complement components in addition to the removal of CFH-autoantibodies made this the 'gold-standard' for management of aHUS. In the last 4 years the introduction of the complement inhibitor Eculizumab has revolutionised the management of aHUS. In this review we shall discuss the available literature on treatment strategies to date.
authors.affiliation: The Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
authors.forename: Edwin K S
authors.initials: EK
authors.lastname: Wong
authors.forename: Tim H J
authors.initials: TH
authors.lastname: Goodship
authors.forename: David
authors.initials: D
authors.lastname: Kavanagh
mesh_terms: D000911:Antibodies, Monoclonal / Q000009:adverse effects / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized / Q000009:adverse effects / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome; D003165:Complement System Proteins / Q000276:immunology; D019852:Diacylglycerol Kinase / Q000235:genetics; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D006801:Humans; D019683:Penetrance; D010951:Plasma Exchange; D011379:Prognosis
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D003165:Complement System Proteins; C481642:eculizumab; C526464:DGKE protein, human; D019852:Diacylglycerol Kinase
keywords: Complement; Eculizumab; Haemolytic Uraemic Syndrome; Treatment